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Joint Restriction in an Unhappy Teenager P J C Davis, J Hackett, K Johnson, J E Mcdonagh

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Joint Restriction in an Unhappy Teenager P J C Davis, J Hackett, K Johnson, J E Mcdonagh 1786 Ann Rheum Dis: first published as 10.1136/ard.2005.036715 on 11 November 2005. Downloaded from LETTERS Joint restriction in an unhappy teenager P J C Davis, J Hackett, K Johnson, J E McDonagh ............................................................................................................................... Ann Rheum Dis 2005;64:1786–1787. doi: 10.1136/ard.2004.034934 14 year old girl presented with a year’s history of joint (17.5 mg orally weekly) while maintaining her prednisolone pain, restriction, and swelling, initially diagnosed as at 3 mg daily. Over the following 6 months finger movement Ajuvenile idiopathic arthritis. She had no past medical improved despite stopping prednisolone in the interim. problems but had given up her leisure pursuits owing to However, she attempted suicide by taking an overdose of disability. Examination disclosed non-pitting oedema to mid- methotrexate as she felt a burden to others, and required calf; a woody texture of the soft tissues of the forearms and further psychological treatment. calves with fixed flexion deformities of her fingers, wrists, Three years after diagnosis, she remains well and continues knees, and ankles due to the soft tissue changes, and normal to receive methotrexate, with minimal inflammation on MRI, overlying skin. Investigations showed the following results: mild finger and wrist contractures, and return of full normal muscle enzymes, eosinophilia of 1.76109/l, erythro- function. At no time has there been evidence of internal cyte sedimentation rate 41 mm/1st h, haemoglobin 112 g/l, organ involvement. negative autoantibodies, and normal complement levels. The EF is characterised by painful swelling and stiffness of the differential diagnosis included eosinophilic fasciitis (EF) and extremities, typically with thickened skin (peau d’orange), a scleroderma. At this time, the patient requested counselling peripheral eosinophilia, and characteristic histology.1–4 In as she had difficulty accepting an initial misdiagnosis, contrast with adult EF, paediatric EF predominantly affects delayed correct diagnosis, and the uncertainty of the girls and has not been reported to cause haematological prognosis. complications. In contrast with scleroderma, the epidermis Magnetic resonance imaging (MRI) of the forearm was and dermis are spared, whereas in comparison with performed (fig 1). A biopsy showed chronic inflammatory polymyositis there is minimal muscle inflammation, distinc- 1–4 changes in the deep subcutaneous fat and fascia with tions illustrated by MRI. infiltration by lymphocytes, plasma cells, histiocytes, and Teenage self harm is well recognised, particularly in girls, although may be underdiagnosed in those with a chronic eosinophils. The fascia was markedly thickened and fibrosed 5 with focal fibrinoid necrosis, consistent with EF. illness. The impact of the disease itself, its impact upon body In view of her disability, she was treated with intravenous image and lifestyle, and the side effects of treatment, lack of predictability, and uncertainty of prognosis are all likely to methylprednisolone (1 g/day for 3 days), then oral predniso- contribute to mental health status during adolescence.6 lone 20 mg daily (0.3 mg/kg/day) with normalisation of her Prognosis in paediatric EF remains uncertain, although a erythrocyte sedimentation rate and eosinophilia. Methotrexate previous study has reported more favourable outcomes in 15 mg orally weekly was added 2 months later as there had 4 older children. We expect to withdraw methotrexate from http://ard.bmj.com/ been no further significant clinical response to steroids. this girl within the next 12–18 months if her signs and MRI Five months later she improved functionally with reduced appearance remains stable. This case highlights the use of contractures despite prednisolone reduction to 12.5 mg daily. MRI in assessment of soft tissue disease and in guiding drug A repeat MRI scan showed a reduction in the subcutaneous management. Furthermore, the psychological impact during and fascial oedema, prompting a further reduction in adolescence of a chronic disease, its management, and correct steroids. At this time, she self harmed by cutting the skin diagnosis should not be underestimated. of her forearms because of low mood, difficulties adjusting to her diagnosis, and the cosmetic side effects of steroids. She JEMcD is an Arthritis Research Campaign clinical senior lecturer. on September 26, 2021 by guest. Protected copyright. required treatment with antidepressants and counselling. One year after diagnosis, an MRI scan showed persistent ..................... fascial oedema, leading to an increase in methotrexate Authors’ affiliations P J C Davis, J Hackett, Department of Rheumatology, Birmingham Children’s Hospital, UK K Johnson, Department of Radiology, Birmingham Children’s Hospital, UK J E McDonagh, Institute of Child Health, University of Birmingham, Birmingham, UK Correspondence to: Dr J E McDonagh, Institute of Child Health, Birmingham Children’s Hospital and University of Birmingham, Steelhouse Lane, Birmingham B4 6NH, UK; [email protected] Accepted 23 April 2005 REFERENCES 1 De Clerck LS, Degryse HR, Wouters E, Van Offel JF, De Schepper AM, Martin JJ, et al. Magnetic resonance imaging in the evaluation of patients with eosinophilic fasciitis. J Rheumatol 1989;16:1270–3. 2 Liou CH, Huang GS, Taylor JAM, Juan CJ, Gao HW, Chen CY. Eosinophilic Figure 1 Coronal short t inversion recovery (STIR) image of arm. The fasciitis in a military recruit: MRI evaluation with clinical correlation. Skeletal white arrows indicate areas of high signal oedema within the fascia. Radiol 2003;32:52–7. www.annrheumdis.com Letters 1787 Ann Rheum Dis: first published as 10.1136/ard.2005.036715 on 11 November 2005. Downloaded from 3 Cassidy JT, Petty RE. Textbook of pediatric rheumatology, 4th ed. 5 Hawton K, Rodham K, Evans E, Weatherall R. Deliberate self-harm in Philadelphia: Saunders, 2001. adolescents: self report survey in schools in England. BMJ 4 Farrington ML, Haas JE, Nazar-Stewart V, Mellins E. Eosinophilic fasciitis in 2002;325:1207–11. children frequently progresses to scleroderma-like cutaneous fibrosis. 6 Suris JC, Michaud PA, Viner R. The adolescent with a chronic condition. Part I: J Rheumatol 1993;20:128–32. Developmental isssues. Arch Dis Child 2004;8:938–42. Relapse of autoimmune diseases after autologous T cell depleted stem cell transplantation may be triggered by T cells recently emigrated from the thymus IKo¨tter*, T Daikeler*, H Einsele, S Koch, L Lochmann, L Kanz, J Lo¨ffler ............................................................................................................................... Ann Rheum Dis 2005;64:1787–1789. doi: 10.1136/ard.2004.032870 igh dose immunosuppressive therapy with subsequent (TRECs) were measured by real time polymerase chain autologous stem cell transplantation (ASCT) has reaction using the light cycler (Roche Diagnostics proved effective for treatment resistant autoimmune Corporation, Idaho, USA). The study was approved by the H 1 diseases. However, in a number of patients, relapses of local ethics committee and patients’ informed consent autoimmune disease after initial improvement or even obtained. remission occur. Until recently, it was impossible to All patients improved considerably (all patients with SSc) differentiate recent thymic emigrants from residual or or went into remission (PsA, WG) after ASCT. In all patients, peripherally expanded T cells. Douek and coworkers2 and previous immunosuppressive therapy was discontinued, later our group3 described a method for detection of T cell except in the patient with WG, who still received ciclosporin receptor excision circles, stable DNA episomes which are A for her kidney transplant. Two patients relapsed after 7, formed during T cell receptor rearrangement in the thymus and 19 months, respectively (one SSc, one PsA). CD3+/CD4+ which are not replicated during mitosis, but are diluted T cells were recovered after 6–14 months (median 12), and during cell divisions.4 This study aimed at investigating CD3+/CD8+ T cells a maximum of 3 months after ASCT in all whether these flares are associated with de novo T cell patients. Autoantibodies disappeared in four of five patients development. after a median of 6 months and their recurrence or Peripheral blood mononuclear cells were collected from six disappearance later did not correlate with relapse or patients, mean age 32.5 years, range 24–42 (four with remission. TRECs could not be detected in the patients systemic sclerosis (SSc), one with Wegener’s granulomatosis shortly after ASCT (week 2), but recurred 9–26 months (WG), one with mutilating psoriatic arthritis (PsA)), after (median 16 months) after ASCT. In the two patients who high dose immunosuppression with cyclophosphamide experienced relapses, TRECs became detectable at the time http://ard.bmj.com/ 50 mg/kg body weight plus anti-thymocyte globulin 20 mg/ when the first symptoms of relapse were noticed (SSc, kg on days 1–4, with subsequent retransfusion of 4.26106 patient No 3: relapse after 7 months, TRECs after 9 months; (median) CD34+ selected (by CliniMacs device) cells/kg body PsA, patient no 4: relapse after 19, TRECs after 21 months— weight. Blood collection was performed at week 2 and then, the blood collection did not always coincide exactly with the at bimonthly intervals, genomic DNA was extracted using the diagnosis of relapse, which explains the 2 months’ difference QIAmp DNA Blood
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